亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯(lián)系我們
强行糟蹋人妻HD中文字幕,内射无码AV-区二区在线观看,久久99蜜桃精品久久久久小说
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-OTC/Ornithine Carbamoyltransferase/Cy5 Conjugated antibody (bs-18037R-Cy5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-18037R-Cy5
英文名稱 Rabbit Anti-OTC/Ornithine Carbamoyltransferase/Cy5 Conjugated antibody
中文名稱 Cy5標記的鳥氨酸氨甲?;€粒體抗體
別    名 EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  神經(jīng)生物學  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 36kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]

Subcellular Location:
Mitochondrion matrix.

Tissue Specificity:
Mainly expressed in liver and intestinal mucosa.

Post-translational modifications:
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.

DISEASE:
Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

Similarity:
Belongs to the ATCase/OTCase family.

Database links:

Entrez Gene: 280887 Cow

Entrez Gene: 480878 Dog

Entrez Gene: 5009 Human

Entrez Gene: 18416 Mouse

Entrez Gene: 397438 Pig

Entrez Gene: 25611 Rat

Entrez Gene: 101102207 Sheep

Omim: 300461 Human

SwissProt: Q9N1U7 Cow

SwissProt: P00480 Human

SwissProt: P11725 Mouse

SwissProt: O19072 Pig

SwissProt: P00481 Rat

SwissProt: P84010 Sheep

Unigene: 117050 Human

Unigene: 2611 Mouse

Unigene: 2391 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 a6308.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
精品国产乱码久久久久久毛片 | 欧美一级二级三级 | 上海少妇高潮狂叫喷水了 | 91人妻无码精品一区二区毛片 | 中文国产高清综合乱色视频在线播放 | 国产日韩精品一区二区三区在线 | 久久精品九九亚洲精品 | 国产69精品久久久久APP下载 | 欧美人成国产91视频 | 欧美一级二级三级 | 国产一区二区三区内射高清 | 国产欧美日韩精品一区二区三区蜜桃不卡 | 成人毛片100免费观看 | 九色91精品国产网站 | 国产亚洲tv在线观看 | 午夜亚洲WWW湿好大 欧美又大又粗又硬又色A片 | 白丝初音未来被调教出奶水 | 中国一级毛片视频 | 欧美人妻AⅤ中文字幕 | 精品国产乱码久久久EA7 | 少妇被黑人到高潮喷出白浆 | 亚洲国产精品成人AV无码久久综合网 | 精品无码国产AV一区二区性色 | 91精品国产自产在线观看 | 激情欧美日韩一区二区 | 亚欧洲乱码视频一二三区 | 欧美性受XXXX黑人XYX性爽 | 亚洲精品欧美精品日韩精品 | 国产成人综合精品久久 | 456亚洲人成影院在线观 | 三上悠亚激情AV一区二区三区 | 好吊妞色视频在线观看 | 精品国产天堂综合一区在线 | 最火的app排行榜前十名 | 在线观看亚洲一区二区 | 亚洲一区在线播放 | 欧美韩国人成网站中文字幕 | 国内久经典AAAAA片 | 久久久久久久国产免费看 | 国产麻豆乱子伦午夜视频观看 | 水野优香vagu224在线播放 |